Perivascular epithelioid cell tumors (PEComas) are rarely found in the urinary tract. The clinicopathologic characteristics of 10 cases, retrospectively collected from 5 medical institutions in 3 different European countries, are presented in this study. Male/female ratio was 3:7 and the average age at diagnosis was 62.7 years. Nine cases were sporadic and 1 showed germline mutation of the TSC2 gene. Eight cases were located in the kidney, 1 in the left adrenal and 1 in the right ureter. All of the patients were alive and free of disease at the time of last contact (mean follow-up, 14.1 mo). Four cases displayed a conventional morphology and 6 showed a prominent sclerotic stroma. By immunohistochemistry, melanocytic markers were consistently expressed, especially HMB-45 (10 cases), MiTF (9 cases), and Melan-A (6 cases). Desmin was expressed in 6 cases; 2 cases were positive for CD117; a single case showed TFE3 expression. pMAPK, mTOR, and pAKT demonstrated variable immunostaining with focal positivity in 7, 4, and 2 cases, respectively. Cytokeratins were repeatedly negative in all cases. PEComas in the urinary tract, especially in the renal region, may show a relatively high frequency of the sclerosing histologic subtype. Knowledge of the distinct histology and immunohistochemical profile is vital to correctly diagnose this rare entity.
- MeSH
- angiomyolipom genetika patofyziologie MeSH
- benigní fibrózní histiocytom genetika patofyziologie MeSH
- cystická onemocnění ledvin genetika patofyziologie MeSH
- dospělí MeSH
- imunohistochemie MeSH
- interferon gama nedostatek genetika MeSH
- kinasy ribozomálního proteinu S6, 70-kDa metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- močové ústrojí patofyziologie MeSH
- nádory z perivaskulárních epiteloidních buněk genetika patofyziologie MeSH
- retrospektivní studie MeSH
- senioři MeSH
- signální transdukce genetika MeSH
- TOR serin-threoninkinasy genetika metabolismus MeSH
- transkripční faktory BHLH-Zip genetika metabolismus MeSH
- tuberózní skleróza genetika patofyziologie MeSH
- urologické nádory genetika patofyziologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
